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Notizen: Background: The indications for operative intervention for symptomatic hypertrophic obstructive cardiomyopathy (HOCM) in infancy and childhood are not well defined because of the rarity of the lesion. The traditional surgical procedure consists of septal myectomy. In an attempt to further improve the outcome of HOCM associated with concentric left ventricular hypertrophy and aortic valve disease in infancy, we have combined resection of the left ventricular septum and free wall with a Ross-Konno procedure. Methods: Three infants (aged 3, 4, and 10 months) with HOCM (left ventricular aortic gradients of 75, 95, and 110 mmHg), associated concentric left ventricular hypertrophy, and valvar aortic stenosis (n = 1) or combined valvar aortic stenosis and regurgitation (n = 2) underwent extensive resection of fibroelastosis and subendocardial myocardium of the left ventricular septum and free wall in combination with a Ross-Konno operation. All three patients had marked systolic anterior motion of the mitral valve. The length of the incision into the ventricular septum was 1.8, 2.0, and 2.3 cm. Results: In all three patients this procedure resulted in a marked reduction of width of the left ventricular septum (median 9 mm vs 14 mm preoperatively) and the left ventricular posterior free wall (median 8 mm vs 12 mm preoperatively) and an almost twofold increase of the left ventricular end-diastolic volume (median 13.5 cm3 vs 7.0 cm3 preoperatively). The neo-aortic valve functioned normally. Systolic anterior motion of the anterior leaflet of the mitral valve had completely resolved in two patients and had markedly regressed in the remaining patient. At follow-up of 15, 17, and 26 months, two patients had absence of a left ventricular outflow tract gradient and the third patient had a residual sub-valvar gradient of 15 mmHg. Conclusions: The reported procedure may be a valuable technique in severe forms of hypertrophic cardiomyopathy associated with aortic valve disease. The operation results in enlargement of the left ventricular stroke volume and improvement of the left ventricular diastolic function, restores aortic valve anatomy and function, and abolishes or decreases systolic anterior motion of the mitral valve.

Notizen: Abstract In repair of coarctation in neonates or young infants, inadequate removal of ductal tissue, failure to address hypoplasia of the aortic arch, and suture line tension have been reported to be important factors of residual or early recurrent stenosis at the coarctation repair site. In a consecutive series of neonates and young infants with coarctation, who were all operated without delay with extended resection, the clinical outcome regarding the development of restenosis and hypertension was studied. In addition, the resected specimens were investigated regarding the completeness of resection of ductal tissue. Twenty-five consecutive neonates and young infants (median age 22 days, range 5 to 39 days) who underwent surgical correction of coarctation were reviewed; the resected specimens were examined histologically to document the extent of ductal tissue in the aortic wall. Fifteen patients had a preductal coarctation with associated cardiovascular anomalies including a hypoplastic aortic arch (n = 11). The remaining 10 patients had a paraductal coarctation without associated intracardiac anomalies. In all patients, the isthmus was bypassed and an end-to-side anastomosis was constructed between the descending aorta and the undersurface of the proximal aortic arch (n = 13) or the distal ascending aorta (n = 12). In 13 patients without marked hypoplasia of tbe aortic arch, the coarctation repair was performed through a left thoracotomy. In the remaining 12 patients, the coarctation was repaired through a median sternotomy with CPB and hypothermic circulatory arrest, on the basis of an associated hypoplastic aortic arch (n = 4), hypoplastic aortic arch with intracardiac anomalies (n = 7), or a “bovine” innominate artery (n = 1). There was no perioperative or late mortality. At a median follow-up of 15 months, 1 patient (4%) developed a recurrent stenosis at the coarctation repair site; in the remaining 24 patients, echocardiograpby showed a widely patent anastomosis with no evidence of a hemodynamically significant gradient. None of the patients had hypertension. Histologic examination of the resected specimens demonstrated the presence of ductal tissue in the descending aorta with maximal extension into its lateral wall (mean 5.2 mm). In all specimens of the paraductal subtype, there was also extension of ductal tissue into the lateral wall of tbe isthmus (mean 3.9 mm). We conclude that: (1) in the absence of marked hypoplasia of the proximal aortic arch, coarctation can be repaired with low mortality and morbidity via a left thoracotomy; (2) in the presence of marked hypoplasia of the proximal aortic arch and/or if associated intracardiac defects also need to be repaired, we advocate repair of the coarctation and associated defects through a median sternotomy with circulatory arrest; (3) in view of the absence of postoperative hypertension in this series, early repair of aortic coarctation is recommended; and (4) because ductal tissue may extend not only into the descending aorta but also into the isthmus, complete excision of the coarctation and bypass of the isthmus are valuable techniques to avoid secondary constriction of the aorta by ductal tissue.

Notizen: Abstract A repair technique is reported for Ebstein's anomaly associated with partial atrioventricular canal. When there is inadequate size of the anterior leaflet of the tricuspid valve, pericardial patch augmentation of the anterior leaflet may result in a well functioning monocusp tricuspid valve.